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Dentinogenesis Imperfecta

An autosomal dominant disorder of tooth development characterized by opalescent dentin resulting in discoloration of the teeth. The dentin develops poorly with low mineral content while the pulp canal is obliterated.
Also Known As:
Capdepont Teeth; Dentinogenesis Imperfecta 1; Dentinogenesis Imperfecta without Osteogenesis Imperfecta; Dentinogenesis Imperfecta, Shields Type 2; Dentinogenesis Imperfecta, Shields Type II; Opalescent Dentin; Opalescent Teeth without Osteogenesis Imperfecta; Dentin, Opalescent; Opalescent Dentin, Hereditary; Teeth, Capdepont; Hereditary Opalescent Dentin
Networked: 134 relevant articles (1 outcomes, 16 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Congenital Abnormalities (Deformity)
2. Megalencephaly
3. Muscle Weakness
4. Contracture
5. Osteogenesis Imperfecta (Lobstein Disease)

Experts

1. Simmer, James P: 7 articles (12/2022 - 05/2007)
2. Hu, Jan C-C: 6 articles (12/2022 - 05/2007)
3. Lu, Yongbo: 4 articles (10/2021 - 04/2004)
4. MacDougall, Mary: 4 articles (01/2017 - 07/2003)
5. Wang, Shih-Kai: 3 articles (12/2022 - 12/2011)
6. Kim, Jung-Wook: 3 articles (10/2021 - 12/2011)
7. Qin, Chunlin: 3 articles (01/2020 - 10/2010)
8. Feng, Jian Q: 3 articles (01/2017 - 04/2004)
9. Yamakoshi, Yasuo: 3 articles (02/2011 - 01/2008)
10. Chan, Hui-Chen: 2 articles (12/2022 - 12/2011)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Dentinogenesis Imperfecta:
1. Alendronate (Alendronate Sodium)FDA LinkGeneric
2. Proteins (Proteins, Gene)FDA Link
01/01/2020 - "The phenotypes we observed in Noggin-overexpressing and Smad4-conditional knockout teeth resemble the phenotype of Dentinogenesis Imperfecta (DGI) type III. Our results show that BMPs regulate post-natal dentinogenesis and that BMP-inhibitory proteins like Noggin play a role in that regulation. "
03/01/2000 - "The non-collagenous dentin matrix proteins are involved in dentinogenesis imperfecta type II (DGI-II)."
09/01/2021 - "A large number of previous studies demonstrated that DSPP is a dentinal-specific protein, and DSPP gene mutations lead to dentin dysplasia and dentinogenesis imperfecta. "
01/01/1994 - "Several biochemical studies have suggested diminished levels of, or even the absence of, this protein, which is associated with the human genetic disease dentinogenesis imperfecta (DGI) type II. However, more recent molecular studies have established that the DPP gene locus is not localized to the region of human chromosome 4 (4q13-q21), where several previous linkage analysis studies have mapped DGI types II and III. The purpose of this study was to determine the presence or absence of DPP in the dentition of a patient affected with DGI type II using a sensitive and specific immunodetection method with a polyclonal antibody against mouse DPP. "
01/01/1992 - "Previous biochemical studies have suggested the absence of this protein associated with the human genetic disease dentinogenesis imperfecta (DGI) Types I and II. However, due to the normal degradation of human DPP during dentin maturation, it has not been possible to establish if these reported differences were due to changes in DPP expression or secondary degradation rates in DGI affected versus normal teeth. "
3. CollagenIBA
4. dentin sialophosphoproteinIBA
5. Amino AcidsFDA Link
6. Nonsense Codon (Nonsense Mutation)IBA
7. Serine (L-Serine)FDA Link
8. PhosphoproteinsIBA
9. Protein Sorting Signals (Signal Peptide)IBA
10. Calcitriol Receptors (Calcitriol Receptor)IBA

Therapies and Procedures

1. Therapeutics
2. Overlay Denture
3. Mouth Rehabilitation
4. Prostheses and Implants (Prosthesis)
5. Tooth Preparation